Stay Current is an audio publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. These podcasts are designed to keep healthcare professionals current while on their commute. Stay Current is created and edited by Todd Ponsky and Nicholas Bruns in partnership with Globalcast MD and is recorded and produced at Akron Children's Hospital in Akron, Ohio. Now it's time for viewer mailbag, and today we have a question from Doctor Scott Ingham for Doctor Mark Levitt regarding his podcast on Hirschprung's disease. Uh, Scott, how are you doing today? Very good. Thanks for, for, uh, calling in. Why don't you tell us a little bit about yourself and then go ahead and, um, ask your, uh, question or give your comments. I'm a pediatric surgeon up here in Fargo, North Dakota with the Stanford Health System, and I appreciate Dr. Levitt's previous couple of GlobalC events with Hirschprung's disease, but there were A couple of areas I think all of us still struggle with that weren't covered in those separate sessions, and I wanted to pose the concern about the algorithm on the use of Botox injections and whether Dr. Levitt, you have certain patients that seem to fit that. Is there a technique difference, a dosage, and some interval concerns that you might want to use. I, I think um that's a great question, and you're right, we didn't um delve deeply into the use of Botox. My, my understanding, frankly, of what actually we're doing with Botox is definitely. Changed in the last couple of years as I've started to collaborate more strongly with GI, and the truth of the matter is I think it has a very valuable role in Hirschberg's disease, but you have to understand what you're trying to do. Botox is paralyzing theoretically the skeletal muscle, but it clearly has some impact on the smooth muscle. And what I believe happens in babies is they have a very tight anal sphincter. No matter what, with or without Hirschprung's disease, and if they hold it in successfully, then in a normal baby they just get constipated, and then the Hirschprung's baby they get enterocolitis. So even after a perfectly done operation, you can have high tone, and by definition, if you do a good job with the surgery and you don't hurt the sphincters and you preserve the 10 or 1 centimeter of the anal canal like you're supposed to. You're going to get some high tone in a baby that doesn't know how to relax. So I think that's the ideal circumstance in the primary situation to do Botox, essentially to overcome their instinct of holding in the stool. Um, so I do use that, and it's for some people talk about actually using it empirically when they've done the primary pull through. I haven't started doing that, but we are designing a prospective trial to test that and see if that would decrease the incidence of enterocolitis. But it's certainly valuable if you have a baby that's coming back with enterocolitis episodes. Um, of course, as we talked about during the podcast, we have to make sure there's no anatomic or pathologic problem with that pull-through. But in the early period, I mean up to one year of age, you can have this bad behavior by the patient, even if it's the perfectly done pull-through. Um, after a year or so, I'm very conscious of the fact that there may be an anatomic or pathologic problem. And those are the patients that I really don't think Botox is very valuable because you have to figure out what the anatomy or pathology problem is. And if you give Botox to those patients, they It might get better temporarily, but you're not giving them a permanent fix. Well, I think, you know, we still struggle with some of those patients that respond to it for that 3 or 4 months that are in that borderline age group that, you know, 6 months to a year and a half, and I guess the question is, is you're seeing an improvement that then goes away, and you've got ganglion cells, or at least what appear to be normal ganglion cells on a rebiopsy if you're concerned about a recurrent Hirschsprung's. And I guess I'm, I'm stuck in that middle group, you know, what is, you know, like you said, it's the anatomical workup, you know, when you see some improvement, you don't have obstruction, you've got ganglion cells, and, um, you know, at what point do you stop doing, you know, the injections, even though they've been helping, and they're over that age group. Well, I think one of the things that I've seen um that has been, is particularly offensive to the ability of the pull through to empty is a retained cuff. And if the cuff is too big or was not split completely or rolled up, You can Botox that. You don't really know that you're actually doing that, but you're actually Botoxing the cuffs, and they will get better temporarily, and they will continue to fall off the wagon every 2 or 3 months. So, if you do a digital rectal exam and do not feel the cuff and do not feel a stricture, And you have biopsied and showed that there are normal ganglion cells, and you have measured the nerves, and that's a really important detail. You can't have nerves that are greater than 40 microns because that is a um a transition zone pull through. And many pathologists are not measuring the nerves, and I want to encourage that all the pediatric surgeons demand that their pathologists do that so that they don't put themselves in a situation where they're doing a beautiful pull-through, but it's the transition zone valve. So if I have a patient that has completely ruled out anatomic and pathologic issues. Including no twist, etc. then I would do Botox. In my experience, that maybe gets done once, maybe a second time, and then you're done. If you're dealing with a patient that's over 1 year of age, And they keep misbehaving. I bet there's an anatomic or pathologic problem. I have yet to meet a patient with Hirschberg's disease, and people think I'm crazy when I say this, but I've yet to meet a patient with Hirschberg's disease that is anatomically perfect. Meaning no stricture, no cuff, no duhamel pouch that's causing trouble, no twist with normal ganglion cells and nerves less than 40 microns. I've never met such a patient that does not spontaneously empty, except the rare patient under a year of age who simply has not learned how to relax their anal canal and allow their poop to exit. So if you're having that happen in an older kid, there is an anatomic or pathologic problem that has just yet not been identified. The actual technique I put in submucosal with a very small gauge needle. I take 100 units of Botox and put it in 10 cc's of saline and put 2.5 cc's submucosal right into the muscle that surrounds the anal canal. And I will never do, ever do. I've never done and will never do an internal sphincterotomy because I'm essentially that's permanent Botox, and my worry is that it could cause permanent incontinence because you shouldn't have to keep Botoxing. They'll eventually figure it out, so why make that a permanent anatomic solution. Now your technique, um, is certainly different than Langer's and, you know, that's been described where he certainly would use a much smaller volume. Is there something that is positive about using that large of a volume? You mean the amount of Botox or the amount of fluid? Well, you're using 100 units, which is not uncommon. Most people do between 60 units and 100 units. And, and are you going 4 different quadrants, and you said splitting up that 1010 mL. Is that correct? Yeah, I put 100 units in 10 cc's and I put 2.5 ccs in each quadrant. This seems like a good amount of fluid to inject. OK. Because that's certainly there, there are people that would go with a lot less volume, and I don't know whether that has a clinical difference. I honestly don't know. You mean that it's more concentrated? I, I don't know. You could, you could certainly use less volume. It doesn't liter of fluid, uh, 1 mL of fluid, and injected 0.25 into each quadrant, and I don't know if the volume has something to do with success rate. I don't know. I don't know. I, I want good spread, but I don't want it to be, to lose its concentration, so. And I've had good results with that, but I've never tried a tiny volume like you're describing. I'm not sure. Uh, that actually leads into my second question that deals with the enlarged pull-through segment that does have normal ganglion cells, and let's say they have normal nerve size. You know, how do we approach those patients because they may have been noncompliant with dilations, they may have been noncompliant with the bowel regimen, and subsequently now they have dilated their pull-through segment. Well, I would say that that is an additional soft indication. Where the bowel segment is dilated. And I'm not sure if that's secondary to all the things you just described, or at the original surgery, the surgeon simply didn't take out enough. They got the good ganglion cells and normal nerves, but they didn't go a little bit higher to get rid of that dilated segment. There are definitely patients that have a dilated segment with no other anatomic abnormality, and they continue to misbehave. And for those patients, I have offered a redo to remove that dilated segment. That's exceedingly rare. Almost always there is something causing the dilation, and that something is either a cuff or a transition zone segment of bowel. Um, but if all things are ruled out, having, and you're still a patient having trouble and there's a dilated segment, then that dilated segment could, could be the explanation. Um, and you're quite right, there are patients that migrate into the systems that have been operated on by others, and you have to make an effort of doing a contrast study, doing examination under anesthesia and a rectal biopsy. And that's a fairly, fairly straightforward workup. And with that, you're going to find a lot of people that are anatomically or pathologically not right, and that's why their poulters are not emptying. Now, have you ever, have you ever tried to save a redo pull through and, and, and benefit from a taper, or do you think you're stuck with the straight pull redo pull through? Well, what I will do is I will redo the pull through. Transanally, saving the anal canal, deliver that up into the abdomen, and then make a decision on whether it needs to be tapered or not. Um, if you do have to taper it, that's going to be a fairly dysmotile segment of bowel for many, many months. In addition, if I ever have to taper or redo, I will always divert that patient with an ileostomy. Um, but these are rare circumstances, what you're describing. Most like, most likely you can remove the dilated segment and bring the healthy segment, uh, down. And most importantly, you have to make sure that you've removed any distal obstruction. I've seen patients that, that have been redone. And the suave cuff was left alone and then I had to redo the redo to remove the cuff, and then the patient got better. They're always tough patients, so. They are, they really are, and so I, I wanna thank both of you, uh, for taking the time to, to hit on these questions because I, I assume that a lot of people are wondering the same thing and, uh, uh, I hope you guys have a good rest of your day. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. You can listen to Stay Current in Pediatric Surgery by subscribing on the iTunes Store or by downloading the Globalcast MD podcast app. Please send questions or comments to us at staycurrent podcast@gmail.com. We'll see you next time.
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